Quadricuspid aortic valve is a rare cardiovascular abnormality. Herein is described the case of a male patient with neurofibromatosis type 1 (NF1) who was found to have a quadricuspid aortic valve causing severe aortic regurgitation. Although congenital cardiac malformations have been described in

patients with NF1, to the best of the present authors’ knowledge this is the first time that a quadricuspid valve in a patient with neurofibromatosis has been described.

The Journal of Heart Valve Disease 2005;14:703-704